Introduction: Currently, no consensus on optimal renal replacement modality has been reached for end-stage renal disease (ESRD) patients complicated with hemophilia. They may require infusion of coagulation factors during each hemodialysis session. In comparison, peritoneal dialysis (PD) might be preferred considering that coagulation replacement is only required for catheter placement. However, limited data on the safety and efficacy of PD for treating ESRD patients with hemophilia were reported.
Methods: This is a single-center retrospective cohort study. ESRD patients diagnosed with hemophilia under PD in Peking Union Medical College Hospital from January 1, 1996 to December 31, 2021 were included and followed-up with every month. Their baseline clinical data, catheter insertion procedure, coagulation factor replacement, complications, and outcome were analyzed and compared with general PD patients.
Results: In total, 8 patients diagnosed with hemophilia were included, all-male, with a mean age of 50.3±13.3 years old. Two were acquired hemophilia A, whereas the rest were hereditary hemophilia A (HHA). Seven patients experienced significant hemoglobin (Hgb) increment after PD. Peritoneal hemorrhage only consisted of a small portion of all hemorrhage. Patients with hemophilia seemed to have lower small solute clearance despite higher baseline peritoneal permeability, and appeared to have increased peritonitis rate than other male PD patients, yet this study is not powered to prove this.
Conclusion: PD is a safe and effective choice for patients with hemophilia and ESRD requiring dialysis. More studies are required to evaluate this certain rare group of patients.
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http://dx.doi.org/10.1016/j.ekir.2022.09.030 | DOI Listing |
Orphanet J Rare Dis
January 2025
Department of Physical and Rehabilitation Medicine, La Paz University Hospital (IdiPaz), Madrid, Spain.
Background: Clinical research has offered many definitions and fragmented perspectives of joint morbidity in haemophilia. As joint damage, pain and mobility impairment can be present without clinical record of persistent bleeding, a person-centric joint morbidity characterisation remained a priority for the haemophilia community, giving rise to the 'problem joint' concept. As diagnosing and managing joint morbidity is critical, the aim of this study was to analyse the holistic burden of problem joints in people with moderate or severe haemophilia A (HA).
View Article and Find Full Text PDFHematology
December 2025
Department of Hematology, The Third People's Hospital of Chengdu, The Affiliated Hospital of Southwest Jiaotong University, Chengdu Second Clinical College of Chongqing Medical University, Chengdu, People's Republic of China.
Objectives: Whether intermediate-dose tertiary prophylaxis can improve quality of life and psychological health in adults with severe/moderate hemophilia A has not been determined. This research aims to explore the impact of intermediate-dose tertiary prophylaxis with recombinant human FVIII (rhFVIII) on quality of life, anxiety and depression in such individuals transitioned from on-demand treatment.
Methods: This retrospective analysis collected data from July 2019 to July 2022.
J Thromb Haemost
January 2025
Amsterdam UMC location University of Amsterdam, Department of Pediatric Hematology, Meibergdreef 9, Amsterdam, The Netherlands. Electronic address:
Persons with hemophilia A (PWHA) lack clotting factor VIII (FVIII) due to a genetic mutation in the F8 gene. The administration of FVIII concentrate leads to the development of neutralizing anti-FVIII antibodies (inhibitors) in about 30% of children with severe hemophilia A. The other 70% of children do not mount a detectable antibody response, suggesting that they may have developed tolerance towards FVIII.
View Article and Find Full Text PDFJ Thromb Haemost
January 2025
Department of Health Sciences, Warwick Medical School, University of Warwick, Coventry, United Kingdom; Department of Geratology, Oxford University Hospitals NHS Foundation Trust, Oxford, United Kingdom.
Background: Same-day emergency care (SDEC) is an expanding area of hospital acute medical care. It aims to minimize delays and manage medical emergency patients within the same day, enabling hospitalization to be avoided; the expectation is that the patients would have required inpatient hospitalization in the absence of the SDEC service. Venous thromboembolism (VTE) prevention is a key medical inpatient safety measure.
View Article and Find Full Text PDFJ Thromb Haemost
January 2025
Centre de Référence de l'Hémophilie, Hospices Civils de Lyon, UR4609 Universite Claude Bernard Lyon 1, Lyon, France. Electronic address:
Background: Efanesoctocog is a B-domain-deleted, Fc-fusion FVIII linked to the D'D3 domain of VWF and two XTEN polypeptides, designed for an ultra-extended half-life for prophylaxis in hemophilia A, but also aiding in managing acute bleeding or surgery in patients on long-term emicizumab. However, no current laboratory method accurately measures FVIII levels in the presence of emicizumab. We hypothesized that the chromogenic (CSA) FVIII assay, specifically calibrated for efanesoctocog using bovine coagulation factors, could provide an accurate assessment of efanesoctocog activity.
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